Background:According to some publications [1] cryoglobulinemic vasculitis in different associated diseases varies.Objectives:To characterize patients with cryoglobulinemic vasculitis (CV) in primary Sjoegren’s syndrome (pSS) and chronic hepatitis C virus (HCV) infectionMethods:54 patients with CV were recruited to this study during 7 years period in our rheumatological center. CV was diagnosed according criteria proposed by Ferri C. [1] and De Vita [2].22 patients had CV associated with HCV (19 women and 3 men) and 32 with pSS (all female). The mean age of patients at the time of inclusion in the study among HCV and pSS patients was 50.4±10.0 years and 55.8±13.5 years, and at CV onset was 42.8± 11.1 years and 47.1±15.3 years (p>0,05).Results:19 patients (86%) with HCV-CV had viremia with prevailed 1b genotype (84%). 8 (42%) patients had severe liver fibrosis (F3-4). SS was diagnosed in 6 (28%) patients with HCV-CV (3 with sSS and 3 with pSS). An increase of aRo antibodies (>50 U / ml) was detected in 3patients, ANA>1/320 - in 5/6 patients. 2 patients with SS were diagnosed MALT-lymphoma of the enlarged salivary and lacrimal glands, and 1 marginal zone lymphoma of the spleen in a patient with HCV-CV without pSS. All patients with lymphoma had type II cryoglobulinemia.Most patients with pSS -CV had late stage and active disease: xerostomia (<0.5 ml/5 min) in 28 (88%), sialoadenitis (>2 foci in MSG), keratoconjunctivitis sicca (in 22 patients, 69%).Lymphomas were diagnosed in 9 patients with pSS-CV (28%). In all cases, they were non-Hodgkin’s lymphomas (NHL): in 7 patients - MALT lymphomas of enlarged parotid salivary (5), lacrimal glands (2), in 5 cases they were accompanied by regional lymph node enlargement. B-cell marginal zone lymphoma of lymph nodes and B-cell large cell lymphoma were observed in 1 case each.No statistically significant differences were found in 2 groups in clinical manifestations of CV (see Graph.1), although patients with pSS had elevated lymphoma rate (28% compared to 6% for HCV, p=0.08), while in HCV-CV a slightly higher incidence of arthritis (22%, p=0.14), glomerulonephritis (56% vs 31% in pSS, p=0.10), and enanthema (19% and 3%, respectively, p=0.06).Type II cryoglobulinemia was detected in 68% cases of HCV-CV and in 72% with pSS, less often mixed polyclonal (27% vs 22%) and oligoclonal (5% and 6%) types. There was in 96% (44/46) cases monoclonal IgM with predominant K-light chain mIg (87%, n=40/46).RF positivity, low C4 complement, increased CRP and the levels of IgG, IgM in 2 groups were almost the same. Obviously pSS-CV group had significantly increased aRo, aLa, ANF titers (p=0.01) and also elevated IgA (p=0.02), lowered CD19+ cell levels (p=0.008) and increased BAFF (p=0.04), while in HCV-CV group high transaminases were typical (p=0.0008). BAFF level (N<0.8 ng/ml) in pSS-CV group was higher (median 2.73 ng / ml (0.66-3.86) than in HCV (median 0.66 ng / ml (0.14 - 0.92), (p=0.04). Patients with pSS-CV had significantly (p=0.008) lowered CD19 + cell count (median 5.3% (2.2-13.6)) if the normal range was 6-19%, in contrast to patients with HCV-associated CV (median 20.5% (13.2-26.0).Graphic 1. Clinical characteristics of HCV and pSS associated CVGraphic 2. Laboratory characteristics of HCV and pSS associated CVConclusion:Clinical picture of CV, as well as the main immunological parameters are similar in patients with associated pSS and HCV. Immunochemical study of serum and urine proteins is required to determine the type of cryoglobulinemia. SS is not rare in HCV-CV, so appropriate examination with ANA, aRo/La detection is mandatory. Patients with mixed monoclonal cryoglobulinemia had increased risk of hematological malignancies.References:[1]Ferri C, Zignego AL, Pileri SA. Cryoglobulins. J Clin Pathol 2002;55:4–13.[2]De Vita S, Soldano F, Isola M, et alPreliminary classification criteria for the cryoglobulinaemic vasculitisAnnals of the Rheumatic Diseases 2011;70:1183-1190.Disclosure of Interests:None declared
IFI16 and Anti-IFI16 as Novel Biomarkers for Sjoegren’s Syndrome: Preliminary Data
